[Influence of Clinical Practice Guidelines on the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis. Data from the Registry of the Spanish Society of Pulmonology and Thoracic Surgery]

Abstract

Objective: The objective of the study was to analyze the diagnostic process and the time until the start of treatment of patients with idiopathic pulmonary fibrosis in relation to the publication of successive clinical practice guide.

Material and methods: Multicenter, observational, ambispective study, in which patients includes in the idiopathic pulmonary fibrosis registry of the Spanish Society of Pulmonologist and Thoracic Surgery were analyzed. An electronic data collection notebook was enabled on the society's website. Sociodemographic and clinical variables were collected at diagnosis and follow-up of the patients.

Results: From January 2012 to december 2019, 1064 patients were included in the registry, with 929 finally analyzed. The diagnosis process varied depending on the year in which it was performed, and the radiological pattern observed in the high-resolution computed tomography. Up to 26.3% of the cases (244) were diagnosed with chest high-resolution computed tomography and clinical evaluation. Surgical biopsy was used up to 50.2% of cases diagnosed before 2011, while it has been used in 14.2% since 2018. The median time from the onset of symptoms to diagnosis was 360 days (IQR 120-720), taking more than 2 years in the 21.0% of patients. A percentage of 79.4 of patients received antifibrotic treatment. The average time from diagnosis to the antifibrotic treatment has been 309 ± 596.5 days, with a median of 49 (IQR 0-307).

Conclusions: The diagnostic process, including the time until diagnosis and the type of test used, has changed from 2011 to 2019, probably due to advances in clinical research and the publication of diagnostic-therapeutic consensus guidelines.

Keywords: Idiopathic pulmonary fibrosis; Interstitial lung disease; Multidisciplinary diagnosis team.

Figura 1

Diagrama de flujo de los pacientes incluidos en el estudio y las causas de su exclusión. *Los diagnósticos alternativos declarados durante el seguimiento de los pacientes fueron: 16 posibles neumonitis por hipersensibilidad crónica, una NINE fibrótica, 4 enfermedades autoinmunes y dudas diagnósticas por falta de progresión de la enfermedad en el paciente 24.

Figura 2

Mediana del tiempo transcurrido desde el inicio de los síntomas hasta el diagnóstico de FPI (días), dividido por el tiempo que transcurre entre la realización de cada una de las técnicas diagnósticas y desde el diagnóstico hasta el comienzo del tratamiento antifibrótico. Las cifras entre paréntesis hacen referencia al rango intercuartílico. Cálculos realizados sobre los 830 pacientes de los que se disponía de información sobre el tiempo desde el inicio de los síntomas hasta el diagnóstico. BIOPSIA: biopsia quirúrgica;LBA: lavado broncoalveolar; Rx tórax: radiografía de tórax; TACAR: tomografía axial computarizada de alta resolución.